Published online Nov 29, 2022. doi: 10.5317/wjog.v11.i4.40
Peer-review started: July 18, 2022
First decision: August 1, 2022
Revised: August 5, 2022
Accepted: November 23, 2022
Article in press: November 23, 2022
Published online: November 29, 2022
Epithelioid hemangioendothelioma (EHE) is an extremely rare tumor with a prevalence of one in a million and a very heterogenous spectrum of disease that varies from an indolent to a metastasizing aggressive disease, with the liver, lung and bone being the primarily involved organs. Peritoneal forms of EHE are even rare, and only 12 cases have been reported to date in the literature.
A 66-year-old female came to our attention complaining low abdominal and perineal pain. Magnetic resonance imaging examination showed a 52 mm × 58 mm × 32 mm cystic mass with some smooth septa, simulating an ovarian cyst. Explorative laparoscopy demonstrated the presence of a peritoneal mass of augmented consistency connected with a sigmoid epiploic appendix in the right side of the Pouch of Douglas, that was surgically removed. Histological exa-mination revealed a primitive peritoneafl hemangioendothelioma. The patient easily recovered from surgery with no residual pain or discomfort. She is regularly attending a 3-years follow-up that is negative for local recurrence of disease or distant metastases.
Peritoneal form of EHE often simulates masses of other nature, as in our case. Given its unspecific clinical and radiological presentation, patients are often forced to a large series of tests and examinations before reaching a definitive diagnosis, that can only histologically made. The possibility of EHE should always be considered in case of unexplained chronic abdominal pain associated to a non-specific mass.
Core Tip: In this article we describe the case of a woman of middle age who came to our observation for chronic abdominal pain. Diagnostic examinations showed a mass of undefined nature which simulated an ovarian cyst due to its pelvic position and its morphological features. Histological examination revealed a primary peritoneal hemangioendothelioma, which is an extremely rare form of epithelioid hemangioendothelioma, with only 12 cases reported in the literature to date. Epithelioid hemangioendothelioma has a very heterogenous spectrum of disease that varies from an indolent to a metastasizing aggressive disease, with the liver, lung and bone being the primarily involved organs.